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Bronchopulmonary sequestration
Bronchopulmonary sequestration Per Holstein and Erik Hjelms, Copenhagen, Denmark
BronchoPulmonary sequestration is an anomaly characterized by partial or complete separation of one segment of the lung from its remaining part. The sequestered lung tissue receives its arterial blood supply through a major, abnormal artery branching directly off the systemic circulation. Two types of bronchopulmonary sequestration have been identified: an extralobar type, in which the sequestered tissue is encased in its own pleural covering, and an intralobar type, in which it is embedded in one of the lobes. Abnormal arterial supply to pulmonary tissue was described as early as 1777. 1 Extralobar sequestration was reported in 1861 2 , 3 and intralobar sequestration in 1946. 4 More than 250 cases are now on record. The diagnosis is frequently difficult because of the motley symptoms and signs. Therefore, we feel justified in contributing to the elucidation of the disease by a brief survey and by submitting 9 cases, 2 of which have been published previously." Pathogenesis
Although an agreement on the pathogenesis has not been reached, most authors believe that the condition is a congenital malformation. At the stage when the embryo is about 8 mm. in length, the fetal anastomoses between the aorta and the pulmonary plexus degenerate. At the same time, foldings and From the Department of Thoracic Surgery R (Department Heads-Ib Andersen and Erik Halkier), The Gentofte Hospital, Copenhagen, Denmark. Received for publication Oct. 3, 1972.
462
branchings take place in the primitive lung primordium, which is derived from the foregut and from which the bronchopulmonary lobes and segments form. In Pryce's' opinion, an aberrant vessel exerts traction on a segment of the pulmonary tissue, so that it becomes sequestered. Supposedly, cysts are then formed as a result of injuries to the pulmonary tissue, which develop in the course of the sequestration. Boyden? was unsure whether the coincidence of two different abnormalities, aberrant vessels and cysts in the lung, was accidental. Smith' advanced the hypothesis that the primitive systemic arterial blood supply is preserved in part of the pulmonary tissue because of insufficient blood supply from the pulmonary artery. The degenerative changes were then supposed to be caused by the high blood pressure in the arterial part of the systemic circulation. These theories are opposed by Gebauer and Mason's" "interpretation of bronchopulmonary sequestration as an acquired disease in which the primary lesion is believed to be an infectious process in the pulmonary tissue, secondarily entailing hypertrophy of bronchial and mediastinal arteries. Pathology
Grossly, the pulmonary tissue is firmly infiltrated and usually has solitary or multiple cysts. The bronchi are dilated, are often filled with mucus or pus, and possibly communicate with cysts. The systemic arterial blood supply is via one or more large arteries, 5 to 20 mm. in diameter. In most
Volume 65 Number 3
Bronchopulmonary sequestration
463
March, 1973
cases, they arise directly from the aorta and course by way of the inferior pulmonary ligament into the sequestration; however, they may also branch off the celiac artery and must then penetrate the diaphragm." 10 The abnormal vessels often exhibit arteriosclerotic changes.' The venous drainage in intralobar sequestration is mainly to the pulmonary veins, whereas in the extralobar type it is to the azygous, hemiazygos, or portal vein.
Clinical features Bronchopulmonary sequestration is most often diagnosed in young adults, rather more often in men than in women. The sex ratio is 1.5: 1 in the intralobar type and 3: 1 in the extralobar type. Bronchopulmonary sequestration may be silent or cause vague symptoms, such as cough and sensations in the chest. In most cases, it manifests itself as bronchitis, bronchiectasis, or recurrent pneumonia. The patients often complain of pain in the chest and sometimes experience hemoptysis. In cases in which bronchopulmonary sequestration causes fistulization between the bronchial tree and the digestive tract, respiratory embarrassment or alimentary symptoms may occur. Thus, by its diverse symptoms and signs, bronchopulmonary sequestration may imitate various diseases, mainly characteristic chronic or recurrent infectious disease of the respiratory tract.
Diagnosis Plain chest radiography shows uncharacteristic infiltration, usually posterobasally in the left lung field. Often, there are cysts and fluid levels. Bronchography may show filling of a cyst and ectatic bronchi. In most cases, however, the bronchi do not fill in the area of the infiltration. The bronchi of adjacent pulmonary areas may exhibit arcuate displacement, with the concavity toward the infiltration. The most important diagnostic measure is thoracic aortography, which may disclose the abnormal arterial supply. Furthermore, this investigation is a valuable aid in planning the operation, as it shows the num-
ber, caliber, and location of the abnormal vessels. Pulmonary angiography may demonstrate absence of pulmonary arteries in the affected area. Other procedures, e.g., bronchoscopy, usually do not afford diagnostic information.
Treatment Extirpation of the bronchopulmonary sequestration is curative. In the intralobar type, this usually necessitates lobectomy, as the adjacent pulmonary tissue often is the seat of chronic inflammatory changes. The systemic arteries must always be isolated and carefully severed and ligated. In the literature there are several reports on operative death caused by exsanguinating hemorrhage from arteries to the bronchopulmonary sequestration."- 12 Their unusual localization led to unintended cutting, whereupon the central, bleeding stump retracted into the mediastinum or below the diaphragm.
Present cases During a period of 8 years, from 1962 to 1970, 9 cases of bronchopulmonary sequestration were diagnosed in the Department of Thoracic Surgery R of the Gentofte Hospital, Copenhagen, Denmark. Table I gives the patients' data, sex ratio, and age distribution. In 7 cases the history was from 6 weeks to 15 years. Two had not had any symptoms of bronchopulmonary sequestration. In 1 such patient, a 6-year-old boy, the anomaly was found in the course of thoracotomy for another disease, a dermoid cyst in the superior mediastinum. In the other patient, an 11-year-old boy who had died of lymphosarcoma, the bronchopulmonary sequestration was an accidential postmortem finding. In 2 patients with symptomatic bronchopulmonary sequestration the diagnosis had been established preoperatively by aortography, and in 3 the diagnosis had been reached on the basis of the symptoms and other radiographic findings. One patient underwent operation because of a diagnosis of bronchiectasis, and 2 had exploratory thoracotomy because of unidentified pulmonary infiltration.
The Journol of
Holstein and Hjelms
464
Thoracic and Cardiovascular Surgery
Table I Case No. (sex, age in years)
Symptoms and signs
Preoperative diagnosis
Most important diagnostic procedure
1 (M, 17)
Cough, expectoration, chest pain, pleuropneumonia
Bronchiectasis
Bronchography, tomography
2 (M, 23)
Cough, expectoration, chest pain, pleuropneumonia
BPS
Thoracic aortography
Retrosternal pain, dysphagia, history of lung abscess
Suspicion of BPS
X-ray films of esophagus showed esophagopulmonary fistula
3 (F, 32) 4 (M, 11)
None
5 (F, 38)
Cough, chest pain, history of empyema
Unidentified pulmonary filtration
6 (F, 19) 7 (F, 5)
Cough, expectoration, history of empyema None
Suspicion of BPS or bronchiectasis Mediastinal tumor
8
(M, 13)
9 (F, 41)
Cough, expectoration, pneuBPS monia, chest pain Chest pain, hemoptysis, history "Vascular anomaly" of pleurisy
In-
Chest radiography. tomography
Bronchography, tomography Chest radiography, tomography
Thoracic aortography Chest radiography, tomography
Legend: BPS, Bronchopulmonary sequestration.
Postoperatively, I patient had a brief spell of respiratory insufficiency which necessitated tracheostomy and respirator treatment. Apart from this, no serious complications occurred. The final diagnosis in all the cases was based upon the finding of fibrous pulmonary tissue devoid of respiratory activity, associated with acute and chronic inflammation. The tissue had dilated, sometimes cystic bronchial remnants with abnormal arteries that branched directly from the systemic circulation. Among the 7 patients with symptomatic bronchopulmonary sequestration, 5 had characteristic histories of recurrent infections and chest pain. A typical history will be reported in brief outline. Case reports 8. D. B. (1924170), a 13-year-old boy, had had repeated attacks of cough and purulent sputum as well as pain in the right chest for the past 6 months. Repeated chest radiography had CASE
shown persisting right-sided basal infiltration. Aortography revealed an abnormal arterial trunk which arose from the aorta and passed directly into the pulmonary infiltration. The venous discharge was not visualized. Pulmonary angiography and pressure measurement in the pulmonary artery showed no abnormalities. Thoracotomy was performed on a diagnosis of bronchopulmonary sequestration. The operation revealed an artery. 8 mm. thick, arising from the anterior aspect of the aorta just above the diaphragm and passing by way of the inferior pulmonary ligament into the lower lobe, which was infiltrated in the basal segments. Moreover, a walnut-sized area of the infiltrated pulmonary tissue extended through the mediastinum into the opposite pleural cavity. The sequestered tissue was resected. Postoperatively. the boy transiently had a partial Horner's syndrome. Microscopic examination of the removed tissue showed acute and chronic inflammatory changes in a highly fibrous pulmonary tissue with ectatic bronchi.
To exemplify the variation in the clinical picture of bronchopulmonary sequestration, the histories of 2 other patients will be reported.
Volume 65
Bronchopulmonary sequestration
Number 3
465
March, 1973
Operative findings
Surgical procedure
Intralobar BPS in the lower lobe with an artery big enough to admit a little finger, that arose from the aorta Intralobar BPS in the lower lobe with an artery big enough to admit a little finger, that arose from the aorta Intralobar BPS in lower lobe with fistula to esophagus and several arteries of subdiaphragmatic origin Extralobar BPS just proximal to diaphragm: lymphosarcoma
Left basal segmental resection
Intralobar BPS with two arteries as thick as a slate pencil, from the angle between the diaphragm, heart. and chest wall Intralobar BPS with an artery big enough to admit a little finger, direct from the aorta Extralobar BPS just above the diaphragm with vascular stalk plus dermoid cyst in superior mediastinum Intralobar BPS with an 8 mm. thick artery direct from the aorta Intralobar BPS with clot-filled cyst; two arteries. 4 and 5 mm. thick, direct from the aorta
Comments
Left lower lobectomy Right lower lobectomy Anterior mediastinotomy
BPS found at autopsy after death from lymphosarcoma (superior vena cava syndrome)
Right lower lobectomy Left lower lobectomy Right basal segmental resection BPS found at thoracotomy done for dermoid cyst in superior mediastinum Right basal segmental resection Left lower lobectomy
CASE 3. L. H. L. N. (252165), a 32-year-old woman, had been treated 7 years previously for pulmonary abscesses in the right lower lobe. She was admitted after having had low retrosternal pain, aggravated on taking solid food, and a sensation of arrest in the esophagus for I year. Chest radiography showed a density inferiorly at the right border of the heart, at the site where 7 years earlier the patient had had several abscess cavities. There were pleural changes, including obliteration of a posterobasal sinus. X-ray films of the esophagus revealed a fistulous duct from the esophagus which passed just above the diaphragm into the affected pulmonary tissue. Bronchography showed irregularly dilated bronchi in the basal segments of the lower lobe and irregular cavities that filled posteriorly. Thoracotomy, done because of a suspicion of bronchopulmonary sequestration, showed inflammatory infiltration and cysts in the lower lobe. There was a fistula to the esophagus distally, on the same level as the esophageal hiatus, and a vascular anomaly in the form of a major artery of subdiaphragmatic origin which entered the affected pulmonary tissue. Lobectomy was performed, the fistula was closed, and the systemic artery was severed and ligated.
Microscopic examination showed dilated bronchi in atelectatic, hyperemic pulmonary tissue with acute and chronic inflammatory changes. In the fistulous duct there was mucosa from the stomach as well as the esophagus. CASE 9. S. I. M. P. (142/71), a 41-year-old woman with a history of left-sided pleurisy 14 years previously, was admitted because of hemoptysis after having suffered from constant pain and a feeling of tenderness in the left chest for some months. X-ray films revealed an infiltration, 2.5 em. in diameter, in the left lower lobe. Two months previously a lung biopsy had shown clots in a cystic cavity. On a suspicion of vascular anomaly, exploratory thoracotomy was carried out. In the left lower lobe there was a cyst, 7 em. in diameter, containing black clots. Two arteries, 5 and 7 mm. in diameter, arose from the anterior aspect of the aorta, about 5 em. above the diaphragm. They entered the tissue surrounding the cyst via the inferior pulmonary ligament. Lobectomy was performed. and the postoperative course was uneventful. Microscopic examination disclosed a cystic wall, without characteristic epithelial cell lining, and lymphocytic infiltration in the surrounding pulmonary tissue.
The Journal of
466
Holstein and Hjelms
Thoracic ond Cordiovascular Surgery
Discussion and conclusion
Summary
Bronchopulmonary sequestration may be asymptomatic or it may manifest itself clinically in extremely different ways, as exemplified by the present cases. The diagnosis may be established by thoracic aortography, but this procedure is done only if bronchopulmonary sequestration is included in the differential diagnostic considerations. Among the 8 patients who underwent operation for bronchopulmonary sequestration, aortography had been done in 2. The large abnormal arteries, which may cause major hemorrhage during the operation, did not pose technical difficulties in any case. The abnormal arterial supply may create arteriovenous shunts. Bjork and colleagues" have described a case of venous drainage to the pulmonary vein in which a flow of 180 ml. per minute was measured in the arteries during the operation. None of our patients exhibited signs of increased cardiac strain. The electrocardiogram was normal in all cases, and cardiac size, judging by radiography in two views, was also normal. In 1 case (Case 1), contrast medium injected into the artery in the operative specimen passed on to the pulmonary vein.'; However, this in vitro investigation cannot be taken as proof of an arteriovenous shunt of hemodynamic significance. The flow of the contrast medium might have been due to injury to the preparation. In another 2 cases in which a similar investigation was performed, there was no shunt in the venous system. The hemodynamic factors, which have been insufficiently elucidated in bronchopulmonary sequestration, should be clarified by selective injection of contrast medium into the abnormal vessels, pressure measurement in the pulmonary circulation, and determination of the cardiac output. Patients with bronchopulmonary sequestration should be treated by operative removal of the sequestered tissue. This will eradicate the chronic infection.
Bronchopulmonary sequestration is briefly reviewed, and 9 cases are submitted. The diagnosis is frequently difficult because of the motley symptoms and signs. A suspicion of bronchopulmonary sequestration indicates that thoracic aortography should be performed, for this study will afford the diagnosis and guidance to the operation. Our patients were not systematically studied for arteriovenous shunt, but this aspect of the disease should be further investigated. REFERENCES
2 3 4 5 6
7 8
9
10 11 12
13
Huber, J. J.: Observationes aliquot de arteria singulari pulmoni concessa, Acta Helvet. 8: 85, 1777. Rokitansky, C.: Lehrbuch der pathologischen Anatomie, ed. 3, Wien, 1861, p. 44. Rektorzik, E.: Ober accessorischen Lungenlappen, Wochenblatt Z. Gesamte Aerzte Wien 17: 4, 1861. Pryce, D. M.: Lower Accessory Pulmonary Artery With Intralobar Sequestration of Lung, J. Patho!. 58: 457, 1946. Nielsen, P. B.: Intralobar Bronchopulmonary Sequestration. Am. J. Roentgeno!. Radium The!'. Nuc!. Med. 91: 547, 1964. Boyden, E. A., Bill, A. H., Jr., and Creighton, S. A.: Presumptive Origin of a Left Lower Accessory Lung From an Esophageal Diverticulum, Surgery 51: 323, 1962. Smith, R. A.: Theory of Origin of Intralobar Sequestration, Thorax 11: 10, 1956. Gebauer, P. W., and Mason, C. B.: Intralobar Pulmonary Sequestration Associated With Anomalous Pulmonary Vessels: A Nonentity, Chest 30: 282, 1959. Gerle, R. D.• Jaretzki, A., Ashley, C. A., and Berne, A. S.: Congenital BronchopulmonaryForegut Malformation, N. Eng!. J. Med. 178: 1413, 1968. Gabriele, O. F.: Arterial Supply to the Lung Via the Celiac Axis, Am. J. Roentgeno!. Radium Ther. NucI. Med. 109: 522, 1970. Douglass, R.: Anomalous Pulmonary Vessels, J. THORAC. SURG. 17: 712, 1948. Harris, H. A.. and Lewis, I.: Anomalies of the Lungs With Special Reference to the Danger of Abnormal Vessels in Lobectomy, J. THoRAC. SURG. 9: 666, 1940. Bjork, L., Dahlstrom, G., and HaIl'en, A.: Pulmonary Sequestration, Scand. J. Resp. Dis. 49: 15, 1968.
BronchoPulmonary sequestration is an anomaly characterized by partial or complete separation of one segment of the lung from its remaining part. The sequestered lung tissue receives its arterial blood supply through a major, abnormal artery branching directly off the systemic circulation. Two types of bronchopulmonary sequestration have been identified: an extralobar type, in which the sequestered tissue is encased in its own pleural covering, and an intralobar type, in which it is embedded in one of the lobes. Abnormal arterial supply to pulmonary tissue was described as early as 1777. 1 Extralobar sequestration was reported in 1861 2 , 3 and intralobar sequestration in 1946. 4 More than 250 cases are now on record. The diagnosis is frequently difficult because of the motley symptoms and signs. Therefore, we feel justified in contributing to the elucidation of the disease by a brief survey and by submitting 9 cases, 2 of which have been published previously." Pathogenesis
Although an agreement on the pathogenesis has not been reached, most authors believe that the condition is a congenital malformation. At the stage when the embryo is about 8 mm. in length, the fetal anastomoses between the aorta and the pulmonary plexus degenerate. At the same time, foldings and From the Department of Thoracic Surgery R (Department Heads-Ib Andersen and Erik Halkier), The Gentofte Hospital, Copenhagen, Denmark. Received for publication Oct. 3, 1972.
462
branchings take place in the primitive lung primordium, which is derived from the foregut and from which the bronchopulmonary lobes and segments form. In Pryce's' opinion, an aberrant vessel exerts traction on a segment of the pulmonary tissue, so that it becomes sequestered. Supposedly, cysts are then formed as a result of injuries to the pulmonary tissue, which develop in the course of the sequestration. Boyden? was unsure whether the coincidence of two different abnormalities, aberrant vessels and cysts in the lung, was accidental. Smith' advanced the hypothesis that the primitive systemic arterial blood supply is preserved in part of the pulmonary tissue because of insufficient blood supply from the pulmonary artery. The degenerative changes were then supposed to be caused by the high blood pressure in the arterial part of the systemic circulation. These theories are opposed by Gebauer and Mason's" "interpretation of bronchopulmonary sequestration as an acquired disease in which the primary lesion is believed to be an infectious process in the pulmonary tissue, secondarily entailing hypertrophy of bronchial and mediastinal arteries. Pathology
Grossly, the pulmonary tissue is firmly infiltrated and usually has solitary or multiple cysts. The bronchi are dilated, are often filled with mucus or pus, and possibly communicate with cysts. The systemic arterial blood supply is via one or more large arteries, 5 to 20 mm. in diameter. In most
Volume 65 Number 3
Bronchopulmonary sequestration
463
March, 1973
cases, they arise directly from the aorta and course by way of the inferior pulmonary ligament into the sequestration; however, they may also branch off the celiac artery and must then penetrate the diaphragm." 10 The abnormal vessels often exhibit arteriosclerotic changes.' The venous drainage in intralobar sequestration is mainly to the pulmonary veins, whereas in the extralobar type it is to the azygous, hemiazygos, or portal vein.
Clinical features Bronchopulmonary sequestration is most often diagnosed in young adults, rather more often in men than in women. The sex ratio is 1.5: 1 in the intralobar type and 3: 1 in the extralobar type. Bronchopulmonary sequestration may be silent or cause vague symptoms, such as cough and sensations in the chest. In most cases, it manifests itself as bronchitis, bronchiectasis, or recurrent pneumonia. The patients often complain of pain in the chest and sometimes experience hemoptysis. In cases in which bronchopulmonary sequestration causes fistulization between the bronchial tree and the digestive tract, respiratory embarrassment or alimentary symptoms may occur. Thus, by its diverse symptoms and signs, bronchopulmonary sequestration may imitate various diseases, mainly characteristic chronic or recurrent infectious disease of the respiratory tract.
Diagnosis Plain chest radiography shows uncharacteristic infiltration, usually posterobasally in the left lung field. Often, there are cysts and fluid levels. Bronchography may show filling of a cyst and ectatic bronchi. In most cases, however, the bronchi do not fill in the area of the infiltration. The bronchi of adjacent pulmonary areas may exhibit arcuate displacement, with the concavity toward the infiltration. The most important diagnostic measure is thoracic aortography, which may disclose the abnormal arterial supply. Furthermore, this investigation is a valuable aid in planning the operation, as it shows the num-
ber, caliber, and location of the abnormal vessels. Pulmonary angiography may demonstrate absence of pulmonary arteries in the affected area. Other procedures, e.g., bronchoscopy, usually do not afford diagnostic information.
Treatment Extirpation of the bronchopulmonary sequestration is curative. In the intralobar type, this usually necessitates lobectomy, as the adjacent pulmonary tissue often is the seat of chronic inflammatory changes. The systemic arteries must always be isolated and carefully severed and ligated. In the literature there are several reports on operative death caused by exsanguinating hemorrhage from arteries to the bronchopulmonary sequestration."- 12 Their unusual localization led to unintended cutting, whereupon the central, bleeding stump retracted into the mediastinum or below the diaphragm.
Present cases During a period of 8 years, from 1962 to 1970, 9 cases of bronchopulmonary sequestration were diagnosed in the Department of Thoracic Surgery R of the Gentofte Hospital, Copenhagen, Denmark. Table I gives the patients' data, sex ratio, and age distribution. In 7 cases the history was from 6 weeks to 15 years. Two had not had any symptoms of bronchopulmonary sequestration. In 1 such patient, a 6-year-old boy, the anomaly was found in the course of thoracotomy for another disease, a dermoid cyst in the superior mediastinum. In the other patient, an 11-year-old boy who had died of lymphosarcoma, the bronchopulmonary sequestration was an accidential postmortem finding. In 2 patients with symptomatic bronchopulmonary sequestration the diagnosis had been established preoperatively by aortography, and in 3 the diagnosis had been reached on the basis of the symptoms and other radiographic findings. One patient underwent operation because of a diagnosis of bronchiectasis, and 2 had exploratory thoracotomy because of unidentified pulmonary infiltration.
The Journol of
Holstein and Hjelms
464
Thoracic and Cardiovascular Surgery
Table I Case No. (sex, age in years)
Symptoms and signs
Preoperative diagnosis
Most important diagnostic procedure
1 (M, 17)
Cough, expectoration, chest pain, pleuropneumonia
Bronchiectasis
Bronchography, tomography
2 (M, 23)
Cough, expectoration, chest pain, pleuropneumonia
BPS
Thoracic aortography
Retrosternal pain, dysphagia, history of lung abscess
Suspicion of BPS
X-ray films of esophagus showed esophagopulmonary fistula
3 (F, 32) 4 (M, 11)
None
5 (F, 38)
Cough, chest pain, history of empyema
Unidentified pulmonary filtration
6 (F, 19) 7 (F, 5)
Cough, expectoration, history of empyema None
Suspicion of BPS or bronchiectasis Mediastinal tumor
8
(M, 13)
9 (F, 41)
Cough, expectoration, pneuBPS monia, chest pain Chest pain, hemoptysis, history "Vascular anomaly" of pleurisy
In-
Chest radiography. tomography
Bronchography, tomography Chest radiography, tomography
Thoracic aortography Chest radiography, tomography
Legend: BPS, Bronchopulmonary sequestration.
Postoperatively, I patient had a brief spell of respiratory insufficiency which necessitated tracheostomy and respirator treatment. Apart from this, no serious complications occurred. The final diagnosis in all the cases was based upon the finding of fibrous pulmonary tissue devoid of respiratory activity, associated with acute and chronic inflammation. The tissue had dilated, sometimes cystic bronchial remnants with abnormal arteries that branched directly from the systemic circulation. Among the 7 patients with symptomatic bronchopulmonary sequestration, 5 had characteristic histories of recurrent infections and chest pain. A typical history will be reported in brief outline. Case reports 8. D. B. (1924170), a 13-year-old boy, had had repeated attacks of cough and purulent sputum as well as pain in the right chest for the past 6 months. Repeated chest radiography had CASE
shown persisting right-sided basal infiltration. Aortography revealed an abnormal arterial trunk which arose from the aorta and passed directly into the pulmonary infiltration. The venous discharge was not visualized. Pulmonary angiography and pressure measurement in the pulmonary artery showed no abnormalities. Thoracotomy was performed on a diagnosis of bronchopulmonary sequestration. The operation revealed an artery. 8 mm. thick, arising from the anterior aspect of the aorta just above the diaphragm and passing by way of the inferior pulmonary ligament into the lower lobe, which was infiltrated in the basal segments. Moreover, a walnut-sized area of the infiltrated pulmonary tissue extended through the mediastinum into the opposite pleural cavity. The sequestered tissue was resected. Postoperatively. the boy transiently had a partial Horner's syndrome. Microscopic examination of the removed tissue showed acute and chronic inflammatory changes in a highly fibrous pulmonary tissue with ectatic bronchi.
To exemplify the variation in the clinical picture of bronchopulmonary sequestration, the histories of 2 other patients will be reported.
Volume 65
Bronchopulmonary sequestration
Number 3
465
March, 1973
Operative findings
Surgical procedure
Intralobar BPS in the lower lobe with an artery big enough to admit a little finger, that arose from the aorta Intralobar BPS in the lower lobe with an artery big enough to admit a little finger, that arose from the aorta Intralobar BPS in lower lobe with fistula to esophagus and several arteries of subdiaphragmatic origin Extralobar BPS just proximal to diaphragm: lymphosarcoma
Left basal segmental resection
Intralobar BPS with two arteries as thick as a slate pencil, from the angle between the diaphragm, heart. and chest wall Intralobar BPS with an artery big enough to admit a little finger, direct from the aorta Extralobar BPS just above the diaphragm with vascular stalk plus dermoid cyst in superior mediastinum Intralobar BPS with an 8 mm. thick artery direct from the aorta Intralobar BPS with clot-filled cyst; two arteries. 4 and 5 mm. thick, direct from the aorta
Comments
Left lower lobectomy Right lower lobectomy Anterior mediastinotomy
BPS found at autopsy after death from lymphosarcoma (superior vena cava syndrome)
Right lower lobectomy Left lower lobectomy Right basal segmental resection BPS found at thoracotomy done for dermoid cyst in superior mediastinum Right basal segmental resection Left lower lobectomy
CASE 3. L. H. L. N. (252165), a 32-year-old woman, had been treated 7 years previously for pulmonary abscesses in the right lower lobe. She was admitted after having had low retrosternal pain, aggravated on taking solid food, and a sensation of arrest in the esophagus for I year. Chest radiography showed a density inferiorly at the right border of the heart, at the site where 7 years earlier the patient had had several abscess cavities. There were pleural changes, including obliteration of a posterobasal sinus. X-ray films of the esophagus revealed a fistulous duct from the esophagus which passed just above the diaphragm into the affected pulmonary tissue. Bronchography showed irregularly dilated bronchi in the basal segments of the lower lobe and irregular cavities that filled posteriorly. Thoracotomy, done because of a suspicion of bronchopulmonary sequestration, showed inflammatory infiltration and cysts in the lower lobe. There was a fistula to the esophagus distally, on the same level as the esophageal hiatus, and a vascular anomaly in the form of a major artery of subdiaphragmatic origin which entered the affected pulmonary tissue. Lobectomy was performed, the fistula was closed, and the systemic artery was severed and ligated.
Microscopic examination showed dilated bronchi in atelectatic, hyperemic pulmonary tissue with acute and chronic inflammatory changes. In the fistulous duct there was mucosa from the stomach as well as the esophagus. CASE 9. S. I. M. P. (142/71), a 41-year-old woman with a history of left-sided pleurisy 14 years previously, was admitted because of hemoptysis after having suffered from constant pain and a feeling of tenderness in the left chest for some months. X-ray films revealed an infiltration, 2.5 em. in diameter, in the left lower lobe. Two months previously a lung biopsy had shown clots in a cystic cavity. On a suspicion of vascular anomaly, exploratory thoracotomy was carried out. In the left lower lobe there was a cyst, 7 em. in diameter, containing black clots. Two arteries, 5 and 7 mm. in diameter, arose from the anterior aspect of the aorta, about 5 em. above the diaphragm. They entered the tissue surrounding the cyst via the inferior pulmonary ligament. Lobectomy was performed. and the postoperative course was uneventful. Microscopic examination disclosed a cystic wall, without characteristic epithelial cell lining, and lymphocytic infiltration in the surrounding pulmonary tissue.
The Journal of
466
Holstein and Hjelms
Thoracic ond Cordiovascular Surgery
Discussion and conclusion
Summary
Bronchopulmonary sequestration may be asymptomatic or it may manifest itself clinically in extremely different ways, as exemplified by the present cases. The diagnosis may be established by thoracic aortography, but this procedure is done only if bronchopulmonary sequestration is included in the differential diagnostic considerations. Among the 8 patients who underwent operation for bronchopulmonary sequestration, aortography had been done in 2. The large abnormal arteries, which may cause major hemorrhage during the operation, did not pose technical difficulties in any case. The abnormal arterial supply may create arteriovenous shunts. Bjork and colleagues" have described a case of venous drainage to the pulmonary vein in which a flow of 180 ml. per minute was measured in the arteries during the operation. None of our patients exhibited signs of increased cardiac strain. The electrocardiogram was normal in all cases, and cardiac size, judging by radiography in two views, was also normal. In 1 case (Case 1), contrast medium injected into the artery in the operative specimen passed on to the pulmonary vein.'; However, this in vitro investigation cannot be taken as proof of an arteriovenous shunt of hemodynamic significance. The flow of the contrast medium might have been due to injury to the preparation. In another 2 cases in which a similar investigation was performed, there was no shunt in the venous system. The hemodynamic factors, which have been insufficiently elucidated in bronchopulmonary sequestration, should be clarified by selective injection of contrast medium into the abnormal vessels, pressure measurement in the pulmonary circulation, and determination of the cardiac output. Patients with bronchopulmonary sequestration should be treated by operative removal of the sequestered tissue. This will eradicate the chronic infection.
Bronchopulmonary sequestration is briefly reviewed, and 9 cases are submitted. The diagnosis is frequently difficult because of the motley symptoms and signs. A suspicion of bronchopulmonary sequestration indicates that thoracic aortography should be performed, for this study will afford the diagnosis and guidance to the operation. Our patients were not systematically studied for arteriovenous shunt, but this aspect of the disease should be further investigated. REFERENCES
2 3 4 5 6
7 8
9
10 11 12
13
Huber, J. J.: Observationes aliquot de arteria singulari pulmoni concessa, Acta Helvet. 8: 85, 1777. Rokitansky, C.: Lehrbuch der pathologischen Anatomie, ed. 3, Wien, 1861, p. 44. Rektorzik, E.: Ober accessorischen Lungenlappen, Wochenblatt Z. Gesamte Aerzte Wien 17: 4, 1861. Pryce, D. M.: Lower Accessory Pulmonary Artery With Intralobar Sequestration of Lung, J. Patho!. 58: 457, 1946. Nielsen, P. B.: Intralobar Bronchopulmonary Sequestration. Am. J. Roentgeno!. Radium The!'. Nuc!. Med. 91: 547, 1964. Boyden, E. A., Bill, A. H., Jr., and Creighton, S. A.: Presumptive Origin of a Left Lower Accessory Lung From an Esophageal Diverticulum, Surgery 51: 323, 1962. Smith, R. A.: Theory of Origin of Intralobar Sequestration, Thorax 11: 10, 1956. Gebauer, P. W., and Mason, C. B.: Intralobar Pulmonary Sequestration Associated With Anomalous Pulmonary Vessels: A Nonentity, Chest 30: 282, 1959. Gerle, R. D.• Jaretzki, A., Ashley, C. A., and Berne, A. S.: Congenital BronchopulmonaryForegut Malformation, N. Eng!. J. Med. 178: 1413, 1968. Gabriele, O. F.: Arterial Supply to the Lung Via the Celiac Axis, Am. J. Roentgeno!. Radium Ther. NucI. Med. 109: 522, 1970. Douglass, R.: Anomalous Pulmonary Vessels, J. THORAC. SURG. 17: 712, 1948. Harris, H. A.. and Lewis, I.: Anomalies of the Lungs With Special Reference to the Danger of Abnormal Vessels in Lobectomy, J. THoRAC. SURG. 9: 666, 1940. Bjork, L., Dahlstrom, G., and HaIl'en, A.: Pulmonary Sequestration, Scand. J. Resp. Dis. 49: 15, 1968.